Nature: Complete and durable remission in a patient with life-threatening scleromyxedema treated with high-dose melphalan and BU with auto-SCT A 51-year-old male presented in March of 2002 with progressive and treatment-refractory scleromyxedema for consideration of auto-SCT. He had a history of pruritic ‘hardened shiny lumps’ over diffusely ... Complete and durable remission in a patient with life-threatening scleromyxedema treated with high-dose melphalan and BU with auto-SCT Scleromyxedema, also known as diffuse/generalized and sclerodermoid lichen myxedematosus or Arndt-Gottron disease, is a primary cutaneous mucinosis characterized by a generalized, papular and sclerodermoid, cutaneous eruption that usually occurs in association with monoclonal gammopathy [1].
The localised form has a more favourable course compared to the generalised form, scleromyxoedema (scleromyxedema), which can involve other organs and is sometimes fatal. Localized and disseminated cases are called papular mucinosis or lichen myxedematosus while generalized, confluent papular forms with sclerosis are called scleromyxedema. Scleromyxedema is an idiopathic disease, meaning its exact cause is unknown. It is primarily characterized by generalized papular and sclerodermoid skin eruptions due to the excessive deposition of mucin, a gel-like substance, in the skin.
scleromyxedema, Scleromyxedema is the deposition of mucin as well as fibroblast proliferation with increased collagen within the dermis Scleromyxedema is a rare fibromucinous disorders, with several clinical and pathological overlaps with scleroderma and scleredema. Etiopathogenesis remains uncovered, and no explanation has been provided either for the origin of mucin deposition or for the paraprotein role.
